The epidemiology and current treatment of pediatric choroid plexus carcinoma: An analysis of the national cancer database.
Authors: Xinxin Wu, Michelle Swedek, Julia Griffin, Timothy Dean Malouff, Peter T. Silberstein
Journal: Journal of Clinical Oncology (2022)

e14024 Background: Choroid Plexus Carcinoma (CPC), a subtype of choroid plexus tumors (CPT), is a rare central nervous system neoplasm mostly affecting the pediatric population. The epidemiology of CPC is poorly understood due to the rarity of the disease. By analyzing the National Cancer Database (NCDB), we describe the demographic and social factors that influence the risk and current treatment of the cancer. Methods: We conducted a retrospective cohort analysis utilizing the 2018 National Cancer Database (NCDB) and included patients with Choroid Plexus Carcinoma with a histology-confirmed WHO grade III tumor, between ages 0-99 years (N = 202). Analysis was completed for the frequency of cases, types of treatment received, and overall survival within the pediatric population (age &lt; 18 years, N = 139) and the adult population. Demographic factors were analyzed using Pearson Chi-squared tests, and survival was estimated using Kaplan-Meier curves. Results: The majority of the cases analyzed were white (55.4%), male (54%), and pediatric patients (66.8%, mean age at diagnosis = 15.17 years) with an average Charlson-Deyo Score of 0.24. The mean age at diagnosis of the pediatric population was 2.34 years (min = less than 1 years old/diagnosed in utero, max = 16, std. deviation = 3.84) while the mean age at diagnosis of the adult population was 43.49 years (min = 18, max = 84, std. deviation = 18.23.) The most common primary site for CPC was malignant neoplasm of the cerebral ventricle (87.6%) followed by malignant neoplasm of the brain stem (5.9%). Surgical procedure of the primary site was performed in 92.6% of cases including local excision, subtotal resection, resection, radical resection, partial resection of a lobe of brain, lobectomy, and surgery NOS. Radiation was not used 71.3% of the time, but was more than three times as likely to be used in the adult population after surgery than in the pediatric population after surgery (44.4% vs 14.4%, p value &lt; 0.001). Most patients received chemotherapy (53%) with the administration of chemotherapy higher in the cohort less than 18 years old (69.8% versus 15.9%, p value &lt; 0.001). Multiagent chemotherapy was administered as the first course of therapy as the top chemotherapeutic choice (86%) especially among the pediatric population (88.6%) compared to the adult population (60%) Palliative care was not provided in any of the cases. There was no statistical significance difference between the general survival of CPC between the pediatric and adult population. Conclusions: Historically, CPC is known as a pediatric cancer, but this NCDB study suggests that the frequency of the adult population affected is not negligible (31.2%). There is a difference regarding the current treatment of CPC in the pediatric versus adult population. Further study is needed to determine the impact of treatment and the importance of socioeconomic factors on the survival of CPC.